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3: Paeds Y3/4 - Paediatrics [15] > [40] Acute Lymphoblastic Leukaemia > Flashcards

[40] Acute Lymphoblastic Leukaemia Flashcards

1
Q

What is acute lymphoblastic leukaemia?

A

A type of cancer that starts from lymphocytes in the bone marrow

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2
Q

When does acute lymphoblastic leukaemia present?

A

It can present in adults and children, but is more often diagnosed in younger people

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3
Q

What % of leukaemias in children does acute lymphoblastic leukaemia account for?

A

80%

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4
Q

What is the peak age of onset of acute lymphoblastic leukaemia?

A

2-5 years

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5
Q

What kind of cells are the malignant cells in acute lymphoblastic leukaemia?

A

Lymphoid precursor cells that are arrested in an early stage of development

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6
Q

What causes the arrest of development of lymphoid precursor cells in ALL?

A

An abnormal expression of genes

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7
Q

What can the abnormal expression of genes result from in ALL?

A

Often a result of chromosomal translocations or abnormalities in chromosome number

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8
Q

Why is there a reduction in normal blood cells in ALL?

A

Because the lymphoblasts replace the normal marrow elements, resulting in a decreased production of normal blood cells

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9
Q

What is the result in the reduced production of normal blood cells in ALL?

A
  • Anaemia
  • Thrombocytopenia
  • Neutropenia
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10
Q

Where else in the body can lymphoblasts infiltrate in ALL?

A

Outside the marrow, particularly in the liver, spleen, and lymph nodes

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11
Q

What are the risk factors for ALL?

A
  • Genetic syndromes
  • Identical twin with ALL
  • Significant radiation exposure
  • Prior chemotherapy
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12
Q

Give 2 examples of genetic syndromes that increase the risk of ALL

A
  • Down syndrome

- Li-Fraumeni syndrome

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13
Q

Describe the onset of presentation of ALL

A

In most children it presents insidiously over several weeks, however in some children the illness progresses very quickly

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14
Q

What do the clinical signs of ALL result from?

A

Disseminated disease and systemic ill-health

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15
Q

What causes systemic ill-health in ALL?

A

Infiltration of bone marrow or other organ with leukaemic blast cells

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16
Q

What clinical features result from bone marrow infiltration in ALL?

A
  • Anaemia
  • Neutropenia
  • Thrombocytopenia
  • Bone pain
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17
Q

What symptoms might result from anaemia in ALL?

A
  • Pallor

- Lethargy

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18
Q

What might neutropenia lead to in ALL?

A

Infection

19
Q

What might thrombocytopenia lead to in ALL?

A
  • Bruising
  • Petechiae
  • Nose bleeds
20
Q

What clinical features result from reticulo-endothelial infiltration in ALL?

A
  • Hepatosplenomegaly

- Lymphadenopathy and superior mediastinal obstruction

21
Q

What clinical features result from CNS infiltration in ALL?

A
  • Headaches
  • Vomiting
  • Nerve palsies
22
Q

What clinical features result from testicular infiltration in ALL?

A

Testicular enlargement

23
Q

What general symptoms may be present in ALL?

A
  • Malaise

- Anorexia

24
Q

What investigations should be done in ALL?

A
  • FBC
  • Bone marrow examiantion
  • Clotting screen
25
Q

What might be found on FBC in ALL?

A
  • Low haemoglobin
  • Thrombocytopenia
  • Evidence of circulating leukaemic blast cells
26
Q

What is the role of bone marrow examination in ALL?

A

Essential to confirm diagnosis, and to identify immunological cytogenic characteristics which give useful prognostic information

27
Q

Why is it important to do a clotting screen in ALL?

A

About 10% of patients have DIC at the time of diagnosis, which can present with haemorrhagic or thrombotic complications

28
Q

What are the differential diagnoses of ALL?

A
  • Infectious mononucleosis
  • Acute myeloid leukaemia
  • Lymphoblastic lymphoma
  • Aplastic anaemia
29
Q

What are the categories in management of ALL?

A
  • Induction of remission
  • Intensification
  • Management of CNS involvement
  • Continuation of therapy
  • Treatment of relapse
30
Q

What does remission imply in ALL?

A

Eradication of the leukaemic blasts, and restoration of normal marrow function

31
Q

How is induction of remission achieved in ALL?

A

Combination chemotherapy including steroids is given

32
Q

What rates of remission are achieved using current induction treatment schedules?

A

95%

33
Q

What is involved in intensification of treatment in ALL?

A

A block of intensive chemotherapy can be given

34
Q

What is the purpose of intensification of treatment in ALL?

A

It improves cure rates

35
Q

What is the problem with intensification of treatment in ALL?

A

Increased toxicity

36
Q

What is the problem with traditional chemotherapy in the management of CNS involvement with ALL?

A

Cytotoxic drugs penetrate poorly into the CNS, and so leukaemic cells at this sit may survive effective systemic treatment

37
Q

What is the result of traditional chemotherapy not being very good for the management of CNS involvement?

A

Intrathecal chemotherapy is used to prevent CNS relapse

38
Q

What should be done with patients who have evidence of CNS disease at diagnosis?

A

They should receive additional doses of intrathecal chemotherapy during induction

39
Q

What is involved in continuation of therapy in ALL?

A

Chemotherapy of modest intensity is continued for a relatively long time, up to 3 years from diagnosis

40
Q

What additional medication (to chemotherapy) is given during continuation of therapy for ALL?

A

Co-trimoxazole

41
Q

What is the purpose of co-trimoxazole given in continuation of therapy for ALL?

A

It is given routinely as prophylaxis to prevent Pneumocystitis pneumonia

42
Q

What is involved in the treatment of relapse in ALL?

A

High-dose chemotherapy with or without total body irradiation, followed by bone marrow transplantation

43
Q

What are the complications of ALL?

A
  • Infection

- Tumour lysis syndrome

3: Paeds Y3/4 - Paediatrics [15] (115 decks)

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